• Clinical and Experimental Pediatrics
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• v.56 no.11
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• pp.500-504
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• 2013

Here we present the case of an 11-year-old female patient diagnosed with Caroli syndrome, who had refractory esophageal varices. The patient had a history of recurrent bleeding from esophageal varices, which was treated with endoscopic variceal ligation thrice over a period of 2 years. However, the bleeding was not controlled. When the patient finally visited the Emergency Department, the hemoglobin level was 4.4 g/dL. Transhepatic intrajugular portosystemic shunt was unsuccessful. Subsequently, the patient underwent percutaneous transhepatic variceal obliteration. Twenty hours after this procedure, the patient complained of aphasia, dizziness, headache, and general weakness. Six hours later, the patient became drowsy and unresponsive to painful stimuli. Lipiodol particles used to embolize the coronary and posterior gastric veins might have passed into the systemic arterial circulation, and they were found to be lodged in the brain, kidney, lung, and stomach. There was no abnormality of the portal vein on portal venography, and blood flow to the azygos vein through the paravertebral and hemiazygos systems was found to drain to the systemic circulation on coronary venography. Contrast echocardiography showed no pulmonary arteriovenous fistula. Symptoms improved with conservative management, and the esophageal varices were found to have improved on esophagogastroduodenoscopy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.16 no.4
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• pp.248-253
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• 2013

Purpose: The aim of this study was to compare the efficacy and safety of band ligation and injection sclerotherapy in the endoscopic treatment of children with variceal bleeding. Methods: The study population included 55 children, all of whom were treated at the time of endoscopic diagnosis of esophageal varices at Asan Medical Center, Seoul, Korea, between January 1994 and January 2011. The primary outcomes included initial success rates and duration of hemostasis after endoscopic management (band ligation vs. injectionsclerotherapy). Results: The mean age was $6.7{\pm}5.2$ years and the mean follow-up time was $5.4{\pm}3.7$ years. The most common cause of esophageal varices was biliary atresia. Of 55 children with acute variceal bleeding, 39 had band ligation and 16 had injection sclerotherapy. No differences between groups were observed in terms of the size, location, and presence of red color sign. The success rates of band ligation and sclerotherapy in the control of acute bleeding episodes were 89.7% and 87.5%. The mean duration of hemostasis after endoscopic intervention was $13.2{\pm}25.1$ months. After one year, 19 of 39 patients (48.7%) treated with band ligation and 7 of 16 patients (43.8%) with injection sclerotherapy had experienced rebleeding episodes. Complications after the procedures were observed in 10.3% and 18.8% of children treated with band ligation and injection sclerotherapy. Conclusion: The results of our current study suggest that band ligation and injection sclerotherapy are equally efficient treatments for the control of acute variceal bleeding and prevention of rebleeding.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.4
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• pp.400-406
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• 2019

We report a 12-month-old female infant who had a history of neonatal sepsis with liver micro-abscesses that resolved with intravenous antibiotics during neonatal period. During her neonatal admission period, no umbilical vein catheter was inserted. Also, she did not undergo any abdominal surgeries or had a postnatal history of necrotizing enterocolitis. However, the child developed upper gastrointestinal bleeding in form of hematemesis and melena secondary to esophageal varices at the age of 12 months with an extra-hepatic portal vein obstruction with cavernous transformation and portal hypertension subsequently. The child underwent a successful endoscopic injection sclerotherapy. She is now 20-month-old and has portal hypertension but otherwise asymptomatic. We are proposing the possibility of a delayed-onset portal hypertension as a complication of liver abscess and neonatal sepsis.

• Gut and Liver
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• v.12 no.6
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• pp.704-713
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• 2018

Background/Aims: Gastric varices (GVs) are a major cause of upper gastrointestinal bleeding in patients with liver cirrhosis. The current treatments of choice are balloon-occluded retrograde transvenous obliteration (BRTO) and the placement of a transjugular intrahepatic portosystemic shunt (TIPS). We aimed to compare the efficacy and outcomes of these two methods for the management of GV bleeding. Methods: This retrospective study included consecutive patients who received BRTO (n=157) or TIPS (n=19) to control GV bleeding from January 2005 to December 2014 at a single tertiary hospital in Korea. The overall survival (OS), immediate bleeding control rate, rebleeding rate and complication rate were compared between patients in the BRTO and TIPS groups. Results: Patients in the BRTO group showed higher immediate bleeding control rates (p=0.059, odds ratio [OR]=4.72) and lower cumulative rebleeding rates (logrank p=0.060) than those in the TIPS group, although the difference failed to reach statistical significance. There were no significant differences in the rates of complications, including pleural effusion, aggravation of esophageal varices, portal hypertensive gastropathy, and portosystemic encephalopathy, although the rate of the progression of ascites was significantly higher in the BRTO group (p=0.02, OR=7.93). After adjusting for several confounding factors using a multivariate Cox analysis, the BRTO group had a significantly longer OS (adjusted hazard ratio [aHR]=0.44, p=0.01) and a longer rebleeding-free survival (aHR=0.34, p=0.001) than the TIPS group. Conclusions: BRTO provides better bleeding control, rebleeding-free survival, and OS than TIPS for patients with GV bleeding.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.16 no.1
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• pp.1-9
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• 2013

Variceal bleeding results in significant morbidity and mortality in both children and adults. The guidelines for the management of variceal bleeding are well established in adults but not in children as there have been insufficient pediatric studies of this disorder. In addition, the adult guidelines for treatment of variceal bleeding cannot be applied directly to children as the etiology and natural course of this disease differs between children and adults. Examples of recommended treatments in children include endoscopic variceal ligation as secondary prophylaxis for biliary atresia whereas a meso-Rex shunt operation for extrahepatic portal vein obstruction. In this review, we discuss prophylaxis options and some technical aspects of endoscopic management for variceal bleeding in children.

• Journal of the Korean Society of Radiology
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• v.84 no.1
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• pp.291-297
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• 2023

Isolated left-sided portal hypertension is a rare clinical syndrome caused by splenic veno-occlusive disease. Splenic vein thrombosis and extrinsic compression causes proximal splenic vein hypertension, and the splenic blood flows into the superior mesenteric or portal vein through the upper stomach's collateral vessels, such as the short gastric, coronary, and gastroepiploic veins. Open splenectomy is recommended to treat gastrointestinal bleeding caused by isolated left-sided portal hypertension. Interventional management could be a clinically useful option for selected patients who want to avoid surgical corrections. The report presents two cases of left-sided portal hypertension with gastric variceal bleeding.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.27 no.3
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• pp.264-270
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• 2023

Backgrounds/Aims: Proximal splenorenal shunt (PSRS) is a commonly performed procedure to decompress portal hypertension, in patients with refractory variceal bleed, especially in non-cirrhotic portal hypertension (NCPH). If conventional methods are hindered by any technical or pathological factors, alternative surgical techniques may be required. This study analyzes the effectiveness of various unconventional shunt surgeries performed for NCPH. Methods: A retrospective analysis of NCPH patients who underwent unconventional shunt surgeries during the period July 2011 to June 2022 was conducted. All patients were followed up for a minimum of 12 months with doppler study of the shunt to assess shunt patency, and upper gastrointestinal endoscopy to evaluate the regression of varices. Results: During the study period, 130 patients underwent shunt surgery; among these, 31 underwent unconventional shunts (splenoadrenal shunt [SAS], 12; interposition mesocaval shunt [iMCS], 8; interposition PSRS [iPSRS], 6; jejunal vein-cava shunt [JCS], 3; left gastroepiploic-renal shunt [LGERS], 2). The main indications for unconventional shunts were left renal vein aberration (SAS, 8/12), splenic vein narrowing (iMCS, 5/8), portalhypertensive vascular changes (iPSRS, 6/6), and portomesenteric thrombosis (JCS, 3/3). The median fall in portal pressure was more in SAS (12.1 mm Hg), and operative time more in JCS, 8.4 hours (range, 5-9 hours). During a median follow-up of 36 months (6-54 months), shunt thrombosis had been reported in all cases of LGERS, and less in SAS (3/12). Variceal regression rate was high in SAS, and least in LGERS. Hypersplenism had reversed in all patients, and 6/31 patients had a recurrent bleed. Conclusions: Unconventional shunt surgery is effective in patients unsuited for other shunts, especially PSRS, and it achieves the desired effects in a significant proportion of patients.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.28 no.1
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• pp.48-52
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• 2024

Backgrounds/Aims: Splenectomy is the most frequently performed procedure as definitive management or as part of shunt surgery or devascularization in portal hypertension. Splenectomy is technically challenging because of the frequent coexistence of multiple collateral varices, splenomegaly, poor liver function, and thrombocytopenia. Early arterial ligation and late mobilization (EALDEM) is the traditional method for splenectomy in portal hypertension. Early spleen mobilization offers good control of the hilum. We aim to compare the effect of the early mobilization and delayed arterial ligation (EMDAL) technique with that of the conventional splenectomy technique in patients with portal hypertension. Methods: During the study period from September 2011 to September 2022, 173 patients underwent surgical intervention for portal hypertension at our institution. Among these patients, 114 underwent the conventional method of splenectomy (early arterial ligation and late splenic mobilization) while 59 underwent splenectomy with the EMDAL technique. Demographics were compared between the two groups. Intraoperative and postoperative outcomes were analyzed using the Mann-Whitney test in each group. A minimum follow-up of 12 months was performed in each group. Results: Demographics and type of surgical procedure were comparable in the two surgical method groups. Median blood loss was higher in the conventional group than in the EMDAL method. The median duration of surgery was comparable in the two surgical procedures. Clavien-Dindo grade III/IV complications were reported more frequently in the conventional group. Conclusions: The splenic hilum can be controlled well and bleeding can be minimised with early mobilization and delayed arterial ligation.